One of the most important features of neurofibromatosis is the milk coffee spot (“café au lait”), which occurs in most people with type 1 neurofibromatosis.
Some are present at birth and may change in size, number, and even color with age. Café au lait spots can appear on non-exposed areas, vary in size, have clear borders, are mostly oval or other shapes, do not protrude from the skin, and have normal skin between the discolored spots.
More than 10% of normal people have one or more coffee spots, but if there are more than 6 coffee spots, it should be noticed.